Miss Ellie, whose lopsided tongue stuck out of the side of her mouth, died at her Tennessee home, the Mountain Press reported.
Despite being blind in her final years, the bug-eyed pooch won Animal Planet's World's Ugliest Dog award last year.
Rescued by owner Dawn Goehring when she was seven years old, Miss Ellie ended up enjoying an unlikely career in showbusiness.
She starred in Animal Planet's Dog 101 and was featured in numerous commercials and television programmes.
She was also part of a troupe of dogs that Ms Goehring trained and which performed regularly at the Comedy Barn in Pigeon Forge, Tennessee.
Miss Ellie had been set to travel to California this month to defend her title.
She also did a lot of work for charity, raising about £70,000 for the Humane Society of the Tennessee Valley.
Miss Ellie
Ms Goehring said she is now planning on raising money to open a new animal shelter.
"I am going to make that my driven goal to do in her honour," she told the Mountain Press.
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